About Me

I am a girl who fell in love, got married and wanted to have a regular ol' ordinary family. What I ended up getting was everything but.
This blog chronicles our journey as a family who was blessed with a very special child.
From the day we found out that she had only a 5% chance of living to the present, life has been a true roller coaster of emotions. And most surprisingly, more rewarding than I ever thought possible.
Click here to contact me.

The Harlie Fund

Due to the requests of many caring people, our family set up a legal trust fund, "THE HARLIE FUND" to help with Harlie's care. You may click here for more information or click on the donate button above.

The Harlie Crew

We invite each fan of Harlie to join the following initial crew members and commit to make a monthly contribution to The Harlie Fund.

With Christy's willingness to share, we have worried about, prayed for and rejoiced in the accomplishments of a remarkable girl. The members of The Harlie Crew together show their support by contributing monthly. If you would like to join us, please call Bill Jeffries at (804) 288-1672.

Tuesday, April 21, 2009

For those of you more interested, you can read this very long, medical-heavy entry that talks more about Harlie's heart defects and how her heart is different from a normal heart. For those of you less interested, just skip it.

Here is a list of her heart defects:

1. Congenitally Corrected Transposition of the Great Arteries: Her heart was formed in a mirror image. Meaning what's normally on the right side of the heart, is on the left, and what's normally on the left side, is on the right.

2. VSD: Ventricular Septal Defect: An opening between the left and right ventricles which causes the blue and red blood to mix. In a normal heart there is no opening and the blood is completely separate.

3. Hypoplastic Right Ventricle. Her right ventricle is smaller than it should be.

4. She has two small Superior VenaCavas (SVCs). In a normal heart, there is one, large SVC which carries the blood from your head to the right atrium. It was this defect (combined with defect #3) that prevented the normal "fix" for her CCTGA (called a Double Switch) and lead us to the Fontan.

4. SubaorticStenosis: A narrowing of the passage way from her right ventricle to the aorta (blood going to the body). They said that her blockage is from tissue that has grown over the passage way.

5. Intermittent Second Degree Heart Block: In most cases of CCTGA, the electrical connections in her heart are compromised. Often times, this requires a pacemaker. They anticipated this at her birth and during her first heart surgery at 4 days old, they placed the pacemaker leads for future connection. It is unknown at this time if she will need the pacemaker to be connected after the Fontan.

The Normal Heart

In a normal heart, the right atrium receives blood returning from the body. There is a "pipe" that carries the blood from your head called the Superior VenaCava and a "pipe" that carries the blood from the rest of the body called the Inferior VenaCava. Both of these pipes empty the blood into the right atrium. This blood is low in oxygen (blue).

It flows into the right ventricle, and is then pumped to the pulmonary artery to the lungs. In the lungs it picks up oxygen (red) then flows through the pulmonary veins to the left atrium and into the left ventricle. It is then pumped out of the aorta to the rest of the body.

In Brief:

Blue blood from body (IVC) and head (SVC) go to right atrium, to right ventricle, to pulmonary artery, to lungs, to left atrium, left ventricle, aorta, then body. Repeat. For many, many, many years!

Right now Harlie's blood flows like this:

Blue blood from body (IVC) to left atrium, to left ventricle to right ventricle to aorta, to body (notice NO lungs are mentioned).

And

Blue blood from head (SVC) to lungs, to right atrium, to right ventricle to aorta, to body.

The Bi-lateral, Bi-Directional Glenn

In her 2nd heart surgery (called the Glenn) the surgeon disconnected her SVCs from feeding blood into her left atrium. Then he connected her SVCs to the pulmonary artery, so blood coming from her head would go straight to her lungs to receive oxygen and then would go to her heart to be pumped out to her body.

Basically, in a normal heart there are two ways for the blood to leave the heart - the pulmonary artery (going to the lungs) and the aorta (going to the body) and they leave from two different ventricles. The small ventricle (right) pumps to the lungs and the large ventricle (left) pumps to the body.

In Harlie's heart, there is only one way for the blood to leave her heart - the aorta (going to the body). Because she essentially only has one ventricle because of her large VSD.

The Fontan

In the Fontan procedure, the surgeon will disconnect the IVC (blood coming from her body and feeding into her left atrium) from her left atrium and will connect it directly to her pulmonary artery so that blood coming from her body will go straight to her lungs to receive oxygen, then will go to her heart to be pumped to her body again.

So, her new circulation will go like this:

Blood from body and head will go to pulmonary artery, lungs, right atrium, her one ventricle and out the aorta to her body. Repeat. For many, many years.

The Damus-Kaye-Stansel Procedure (DKS)

The DKS is the creation of a connection (made of donated tissue or Gore-Tex) between her left ventricle to her aorta. It will give the blood another way out of her combined ventricles to the aorta, to her body.

4 comments:

Sarah Gunn
said...

Whew! I am having flashbacks to every biology/anatomy class I ever took, which I barely passed by the way. I am impressed Christy!! I know it's not ideal, but I hope you get to go home today. Good luck and give Harlie and they boys a big hug!

I am exhausted after reading all that...I was just as hard when you explaned it to me and your mom! OK..point is...Dr.Jonas knows what has to be done...can fix it...and we just have to get Harlie healthy..right? You can do this!!!! your the best!

Isn't it amazing that we parents of VACTERL children know more then doctors? Crazy huh? That is a lot of info that's for sure. I think we should be given medical degrees for just having our girls. LOL.

Murphy

Murphy is 13 and is in eighth grade. He is constantly making us laugh with his crazy sense of humor and wonderful imagination. He is a great big brother and adjusted with no problems to this role. He has been an easy kid to raise from the get-go (despite the fact he entered this world 5 weeks ahead of schedule).

Harlie Caroline

Harlie is 10 and is in fourth grade. She has Goldenhar Syndrome, VACTERL Association and a lung defect that required the removal of 2 lobes on the right side. After over 50 surgeries (4 of them open heart), she is remarkably age appropriate. She is the sweetest, funniest little girl I've ever met. You can't help but fall in love with her.

Cooper

Cooper is 8 and is in third grade. He is a very talkative, high energy and affectionate little guy. He is all boy and he is a very funny kid. We are so lucky to have him!

Rooney

Rooney was born on February 5, 2012. He has been a wonderful addition to our family. He is such a great little dog and I just can't imagine life without him now! I love him so much!!!